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PubMed1991

Giant cell fibroblastoma. A case report and immunohistochemical comparison with ten cases of dermatofibrosarcoma protuberans.

A 7-year-old boy with giant cell fibroblastoma (GCF) of the skin and subcutaneous tissue of the right chest wall is described. To date, the histogenesis of GCF has not been clarified. The reason for the diversity of immunohistochemical data among various authors may be because the specimens studied were from only part of the lesion, or reduction of antigenicity through the preparation process. However, our findings based on studies of many specimens from various parts of the tumor for accurate immunohistochemical evaluation suggest that GCF may be a myofibrohistiocytic tumor. Recently, the suggestion that GCF is a juvenile form of dermatofibrosarcoma protuberans (DFSP) has been reported. In addition to the present case, we performed immunohistochemical examination of 10 cases of definitely diagnosed DFSP for comparison. The immunohistochemical characteristics of these two neoplasms were concordant. However, from clinical and morphological viewpoints, it seems premature to recognize GCF as a juvenile form of DFSP.

Child↗
PubMed1981

Dermatofibrosarcoma protuberans of the vulva. Case report.

A woman aged 83 had a vulval swelling measuring 8 cm x 3.5 cm excised. Histological examination showed the features of dermatofibrosarcoma protuberans. This tumour, which is usually only locally invasive, has not previously been described in the vulva.

Aged↗
PubMed1978

Dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans is a well-differentiated sarcoma that rarely metastasizes but is characterized by a high recurrence rate. This is due to clinically undetectable, microscopic extensions into the surrounding dermis. Chemosurgery (Mohs) is effective in the ablation of this tumor because the extensions can be identified and eradicated.

Adult↗
PubMed1985

Chemosurgical reports: myxoid dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans (DFSP) is a fibroblastic tumor characterized by a high rate of recurrence following conventional surgical treatment. Several different histopathologic patterns exist, with the "cartwheel" pattern the most common. In this report, a patient with the unusual myxoid histopathologic pattern was successfully treated with Mohs surgery.

Adult↗
PubMed1987

A ten-year-old boy with dermatofibrosarcoma protuberans of the face.

A rare case of dermatofibrosarcoma protuberans on the face of a 10-year-old boy is presented. The patient's young age and the localization of the tumor led to limited excisions that resulted in two local recurrences. Primary wide excision with 2.5 cm of free margins is recommended to avoid local recurrences that are otherwise frequent.

Child↗
PubMed1990

Dermatofibrosarcoma protuberans in a 9-year-old child: treatment by MOHS micrographic surgery.

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally and deeply recurring dermal neoplasm that, when it occurs in cosmetically sensitive areas, can be devastating, particularly in children. MOHS micrographic surgery is now recognized as the treatment of choice for DFSP. A lesion of DFSP near the breast of a 9-year-old girl was treated by this technique. To our knowledge, this is the first case of DFSP in a child in which MOHS micrographic surgery was used as the first-line treatment modality.

Child↗
PubMed1993

Congenital dermatofibrosarcoma protuberans.

Congenital dermatofibrosarcoma protuberans occurred in a 16-year-old girl. The lesion was a hard cutaneous plaque on the abdomen. It was present at birth and slowly enlarged during the patient's lifetime. Histologic examination was essential to establish the diagnosis and to differentiate it from other congenital fibrohistiocytic proliferations.

Abdomen↗
PubMed1979

Dermatofibrosarcoma protuberans. An ultrastructural study of five cases.

An ultrastructural study of five cases of dermatofibrosarcoma protuberans revealed that the basic cell was a spindle shaped fibroblast with a peculiar deeply lobulated nucleus. An origin from perineural and endoneural cells could not be established since basal lamina material was sparsely present in only one case and other differential features were missing. In one case histiocytic cells were found, but also in this case the basic cell was the fibroblast; so evidence of relationship with fibrous histiocytoma was not found.

Adult↗
PubMed1986

Non-polarizable collagen in dermatofibrosarcoma protuberans: a useful diagnostic aid.

The stroma in dermatofibrosarcoma protuberans (DFSP) stains with trichrome, but is not polarizable (birefringent, doubly refractile). The usefulness of polarizing neoplastic stroma as a diagnostic aid in distinguishing DFSP from dermatofibroma and fibromatosis was evaluated. Forty cases of dermatofibroma of all types, 12 cases of dermal fibromatosis, and 15 cases of DFSP were examined. None of DFSPs contained polarizable collagen, whereas 11 of the 12 (92%) fibromatoses and 35 of the 40 (88%) dermatofibromas did. Of the 5 dermatofibromas that did not polarize, all were composed primarily of round to polyhedral histiocytic cells, including foam cells, frequently surrounding small obliterated capillaries. Although not pathognomonic, the presence of nonpolarizable collagen appears to be an additional histological marker useful in differentiating DFSP from dermatofibroma and fibromatosis.

Collagen↗
PubMed1991

Dermatofibrosarcoma protuberans presenting in infancy and childhood.

Dermatofibrosarcoma protuberans (DFSP) is a not uncommon low-grade cutaneous sarcoma of uncertain histogenesis, which typically arises in early to middle adult life. Traditionally, it is regarded as extremely uncommon in infants and children, and this diagnosis may therefore easily be overlooked in young patients. Eight such cases (representing 5.9% of the available DFSPs on file) are presented of which two were congenital. Age range at presentation was 14 months to 12 years. Five arose on the trunk. Most had originally been mistaken for unclassified sarcoma, a fibromatosis, or diffuse neurofibroma. The histologic features were entirely comparable to the more usual adult cases except that all had a plaque-like, rather than nodular, growth pattern. Short-term follow up has revealed no recurrences. DFSP is not so rare in childhood and warrants wider recognition in order to ensure appropriate treatment.

Age Factors↗
PubMed1992

Dermatofibrosarcoma protuberans: ultrastructural investigation.

The ultrastructural aspects of nucleus and cytoplasm of tumor cells and the interstitial material of dermatofibrosarcoma protuberans are described in detail, especially the very peculiar labyrinthic nucleus. Opinions about its development are reviewed; some, such as melanocytic, neural, histiocytic and smooth muscle proliferation, might be discarded. The myofibroblast appears as the most probable stem cell.

Aged↗
PubMed1992

Pigmented dermatofibrosarcoma protuberans (Bednar tumour).

A case of pigmented dermatofibrosarcoma protuberans in a 37-year-old man is reported. The tumour presented as a nodular lesion located in the left scapular area. Surgical resection with wide margins was performed. No recurrences have appeared to date. The presumptive origin of this entity is briefly discussed.

Adult↗
PubMed1983

Dermatofibrosarcoma protuberans. A clinico-pathological study of nineteen cases and review of world literature.

The clinical course, pathological features and response to treatment in a consecutive 16-years series of 19 cases of dermatofibrosarcoma protuberans were studied. A review of the world literature, comparing the results to ours too was performed. The tumour, occurring at every age and equally among females and males, most commonly involves the trunk without any particular pattern. The disease usually runs an indolent course and is too often regarded lightly by patients as well as physicians leading to delayed and often too conservative treatment. The tumour has definite invasive and metastatic potential and is considered a low grade soft tissue sarcoma. To avoid mutilating surgery (and maybe metastases) later in the course, the initial treatment must be wide surgical excision including a surrounding margin of at least 1 inch normal tissue. The removal of underlying deep fascia is essential and adequate resection will require a skin graft replacement in nearly every instance. Follow up periods should be no less than 3 years at the surgical and oncological service and should be continued at the general practitioners because tumours may recur after considerable periods of time, even in apparently adequately treated patients.

Adult↗
PubMed1981

Dermatofibrosarcoma protruberans of the scalp treated by radical excision, immediate cranioplasty, and free groin flap: case report.

The authors describe a case of dermatofibrosarcoma protruberans of the frontal region in a young girl. The condition was treated by radical excision of the scalp, frontal bone, and sinuses, and immediate reconstruction with an acrylic cranioplasty and microsurgical transfer of a free groin flap. Pathological examination revealed the margins of the excised specimen were clear of tumor. The initial flap failed, but a second graft was immediately transferred successfully, with a good cosmetic result.

Adolescent↗
PubMed1975

[Multiple dermatofibromas with protuberans dermatofibrosarcoma].

A 40 year-old female with multiple dermatofibromas (82) associated with Dermatofibrosarcoma Protuberans is presented. Although of a common fibroblastic origin, these lesions are considered independent. Their presence in the same patient is considered of interest.

Adult↗
PubMed1992

Dermatofibrosarcoma protuberans. A clinicopathologic review with emphasis on fibrosarcomatous areas.

We reviewed 75 cases of dermatofibrosarcoma protuberans (DFSP) from the University of Texas M.D. Anderson Cancer Center. All accessions were examined for areas of giant cell fibroblastoma (GCF), but none was found. The 30 cases having a minimum of 5 years follow-up were studied in more detail. The histologic findings were typical of DFSP in 24 cases, whereas in six cases discrete areas with a fascicular or "herringbone" growth pattern, considered to represent fibrosarcomatous change (DFSP-FS), were evident. The mitotic rate was usually but not always higher in fibrosarcomatous areas, and occasional examples of typical DFSP demonstrated relatively numerous mitotic figures (up to 35 per 10 high-power fields). Other histologic findings of interest were the presence of melanin in two cases of DFSP and the focal presence of a distinctive type of multinucleated giant cell similar to those seen in GCF in six cases. Patients with DFSP-FS differed from those with DFSP in that they had a higher median age (56 years vs. 37 years). Tumor location was similar in both groups, with the trunk being the most common site. No significant difference in either the rate of local recurrence or the interval until recurrence between DFSP and DFSP-FS was evident; the only factor strongly related to local recurrence was adequacy of surgical margins. However, the only two patients who died of tumor, including the sole patient with distant metastasis, had DFSP-FS. We conclude that DFSP-FS deserves recognition as a variant of DFSP.

Adult↗
PubMed1992

Dermatofibrosarcoma protuberans: treatment and prognosis.

Dermatofibrosarcoma protuberans (DFSP) is a rare tumor of the skin with a strong tendency to recur locally. Nineteen cases of DFSP are presented. In eight of them a total of 20 local recurrences occurred, in five after irradical and in three after 'narrow' excisions. After wide excision (greater than 2 cm) for primary or wide re-excision for recurrent tumor, all patients remained free of tumor with a mean follow-up of 13.2 years (range 2-28 years). An extensive literature review revealed 913 cases of DFSP. The overall recurrence rate is about 50%; after adequate wide excision, 13%. Recurrent tumor is safely treated by wide re-excision and the recurrence rate is then 12%. Regional and distant recurrences are infrequent. Eleven cases (1%) were reported to have regional lymph node metastases and 37 (4%, 17 of whom were histologically confirmed) distant metastases, principally in the lung. The prognosis after appearance of regional or distant recurrence is bad. The role of radiotherapy in the management of this tumor is unclear. Primary or recurrent DFSP is best treated by surgical excision with a minimal margin of 2- preferably 3-cm of surrounding skin including the underlying fascia. Elective lymph node dissection is not advised.

Adult↗